An Ultrastructural Study of the Reactive Type of Elastosis Perforans Serpiginosa

Abstract

To the Editor.— Since elastosis perforans serpiginosa (EPS) was first described, it has been suggested that it is a phenomenon caused by a variety of abnormal elastic fibers. At least three etiologic types are now considered to be involved, ie, the idiopathic, the reactive, and the penicillamine-induced. 1-3 Previous ultrastructural investigations showed that the abnormal elastic fibers in the idiopathic type differ in structure from those of the penicillamine-induced type. 1,3-6 The reactive type, which is associated with different congenital disorders, mostly of the connective tissue, such as Down's syndrome, osteogenesis imperfecta, Marfan's syndrome, pseudoxanthoma elasticum, and Ehlers-Danlos syndrome (EDS), has not been as extensively studied. 7 It remains to be demonstrated whether it is a unique structural form of EPS. We performed an ultrastructural study in two patients with the reactive type of EPS. Report of Cases.—Case 1.— A 15-year-old girl presented with osteogenesis imperfecta, type Ia,